Abstract
A number of features of the pathology occurring in spontaneously hypertensive stroke prone rats (SHRSPs), such as MRI brain signal abnormalities, the presence of high protein content in cerebrospinal fluid and vessel wall thickening, seem to indicate that this strain is a suitable model for cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). To explore this hypothesis, we sought the human diagnostic hallmarks of the disease [the accumulation of granular osmiophilic material (GOM) deposits in vessel walls and NOTCH3 gene mutations] in SHRSPs. Male SHRSPs fed a permissive diet were sacrificed 3 days after the first MRI visualisation of brain abnormalities. Whole blood and kidney samples were respectively collected for molecular and electron microscopy evaluations. Automated sequence analysis of exons and intron-exon boundaries did not reveal any genetic variation in the NOTCH3 gene, and electron microscopy excluded the presence of GOM. The findings of this study exclude SHRSPs as a possible model for CADASIL.
| Original language | English |
|---|---|
| Pages (from-to) | 427-430 |
| Number of pages | 4 |
| Journal | Journal of Molecular Neuroscience |
| Volume | 46 |
| Issue number | 2 |
| DOIs | |
| Publication status | Published - Feb 2012 |
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ASJC Scopus subject areas
- Medicine(all)
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Is the SHRPS strain a suitable model of spontaneous CADASIL? / Penco, Silvana; Gelosa, Paolo; Pileggi, Silvana; Abbate, Mauro; Marocchi, Alessandro; Guerrini, Uliano; Pignieri, Alice; Tremoli, Elena; Sironi, Luigi.
In: Journal of Molecular Neuroscience, Vol. 46, No. 2, 02.2012, p. 427-430.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Is the SHRPS strain a suitable model of spontaneous CADASIL?
AU - Penco, Silvana
AU - Gelosa, Paolo
AU - Pileggi, Silvana
AU - Abbate, Mauro
AU - Marocchi, Alessandro
AU - Guerrini, Uliano
AU - Pignieri, Alice
AU - Tremoli, Elena
AU - Sironi, Luigi
PY - 2012/2
Y1 - 2012/2
N2 - A number of features of the pathology occurring in spontaneously hypertensive stroke prone rats (SHRSPs), such as MRI brain signal abnormalities, the presence of high protein content in cerebrospinal fluid and vessel wall thickening, seem to indicate that this strain is a suitable model for cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). To explore this hypothesis, we sought the human diagnostic hallmarks of the disease [the accumulation of granular osmiophilic material (GOM) deposits in vessel walls and NOTCH3 gene mutations] in SHRSPs. Male SHRSPs fed a permissive diet were sacrificed 3 days after the first MRI visualisation of brain abnormalities. Whole blood and kidney samples were respectively collected for molecular and electron microscopy evaluations. Automated sequence analysis of exons and intron-exon boundaries did not reveal any genetic variation in the NOTCH3 gene, and electron microscopy excluded the presence of GOM. The findings of this study exclude SHRSPs as a possible model for CADASIL.
AB - A number of features of the pathology occurring in spontaneously hypertensive stroke prone rats (SHRSPs), such as MRI brain signal abnormalities, the presence of high protein content in cerebrospinal fluid and vessel wall thickening, seem to indicate that this strain is a suitable model for cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). To explore this hypothesis, we sought the human diagnostic hallmarks of the disease [the accumulation of granular osmiophilic material (GOM) deposits in vessel walls and NOTCH3 gene mutations] in SHRSPs. Male SHRSPs fed a permissive diet were sacrificed 3 days after the first MRI visualisation of brain abnormalities. Whole blood and kidney samples were respectively collected for molecular and electron microscopy evaluations. Automated sequence analysis of exons and intron-exon boundaries did not reveal any genetic variation in the NOTCH3 gene, and electron microscopy excluded the presence of GOM. The findings of this study exclude SHRSPs as a possible model for CADASIL.
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U2 - 10.1007/s12031-011-9605-4
DO - 10.1007/s12031-011-9605-4
M3 - Article
VL - 46
SP - 427
EP - 430
JO - Journal of Molecular Neuroscience
JF - Journal of Molecular Neuroscience
SN - 0895-8696
IS - 2
ER -